{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,2]],"date-time":"2026-09-02T10:54:37Z","timestamp":1788346477469,"version":"build-2803163510"},"reference-count":75,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2006,2,16]],"date-time":"2006-02-16T00:00:00Z","timestamp":1140048000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Vox Sanguinis"],"published-print":{"date-parts":[[2006,5]]},"abstract":"<jats:p>Thrombotic thrombocytopenic purpura (TTP), characterized by thrombocytopenia and microangiopathic haemolytic anaemia, was almost universally fatal until the introduction of plasma exchange (PE) therapy in the 1970s. Based on clinical studies, daily PE has become the first\u2010choice therapy since 1991. Recent findings may explain its effectiveness, which may include, in particular, the removal of anti\u2010ADAMTS13 autoantibodies and unusually large von Willebrand factor multimers and\/or supply of ADAMTS13 in acquired idiopathic or congenital TTP. Based on currently available data, the favoured PE regimen is daily PE [involving replacement of 1\u20131\u00b75 times the patient's plasma volume with fresh\u2010frozen plasma (FFP)] until remission. Adverse events of treatment are mainly related to central venous catheters. The potential reduction of plasma related side\u2010effects, such as transfusion\u2010related acute lung injury (TRALI) or febrile transfusion reactions by use of solvent\u2013detergent treated (S\/D) plasma instead of FFP is not established by controlled clinical studies. Uncontrolled clinical observations and the hypothesis of an autoimmune process in a significant part of the patients with acquired idiopathic TTP suggest a beneficial effect of adjunctive therapy with corticosteroids. Other immunosuppressive treatments are not tested in controlled trials and should be reserved for refractory or relapsing disease. There is no convincing evidence for the use of antiplatelet agents. Supportive treatment with transfusion of red blood cells or platelets has to be evaluated on a clinical basis, but the transfusion trigger for platelets should be very restrictive. Further controlled, prospective studies should consider the different pathophysiological features of thrombotic microangiopathies, address the prognostic significance of ADAMTS13 and explore alternative exchange fluids to FFP, the role of immunosuppressive therapies and of new plasma saving approaches as recombinant ADAMTS13 and protein A immunoadsorption.<\/jats:p>","DOI":"10.1111\/j.1423-0410.2006.00747.x","type":"journal-article","created":{"date-parts":[[2006,2,16]],"date-time":"2006-02-16T16:16:09Z","timestamp":1140106569000},"page":"245-254","source":"Crossref","is-referenced-by-count":46,"title":["Treatment of thrombotic thrombocytopenic purpura"],"prefix":"10.1111","volume":"90","author":[{"given":"S.","family":"Fontana","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. A.","family":"Kremer Hovinga","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B.","family":"L\u00e4mmle","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B.","family":"Mansouri Taleghani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2006,2,16]]},"reference":[{"key":"e_1_2_7_2_2","first-page":"21","article-title":"Hyaline thrombosis of the terminal arterioles and capillaries: a hitherto undescribed disease","volume":"24","author":"Moschcowitz E","year":"1924","journal-title":"Proc N Y Pathol Soc"},{"key":"e_1_2_7_3_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra020528"},{"key":"e_1_2_7_4_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V87.10.4223.bloodjournal87104223"},{"key":"e_1_2_7_5_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V87.10.4235.bloodjournal87104235"},{"key":"e_1_2_7_6_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199811263392202"},{"key":"e_1_2_7_7_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199811263392203"},{"key":"e_1_2_7_8_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V98.6.1654"},{"key":"e_1_2_7_9_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.C100515200"},{"key":"e_1_2_7_10_2","doi-asserted-by":"crossref","first-page":"2839","DOI":"10.1182\/blood.V91.8.2839.2839_2839_2846","article-title":"Acquired deficiency of von Willebrand factor\u2010cleaving protease in a patient with thrombotic thrombocytopenic purpura","volume":"91","author":"Furlan M","year":"1998","journal-title":"Blood"},{"key":"e_1_2_7_11_2","doi-asserted-by":"publisher","DOI":"10.1038\/35097008"},{"key":"e_1_2_7_12_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198212023072306"},{"key":"e_1_2_7_13_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2002-02-0344"},{"key":"e_1_2_7_14_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V98.6.1765"},{"key":"e_1_2_7_15_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1537-2995.2002.00100.x"},{"key":"e_1_2_7_16_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2003-01-0193"},{"key":"e_1_2_7_17_2","doi-asserted-by":"publisher","DOI":"10.1053\/j.seminhematol.2003.10.008"},{"key":"e_1_2_7_18_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2003-11-4035"},{"key":"e_1_2_7_19_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V93.11.3798"},{"key":"e_1_2_7_20_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2001.02704.x"},{"key":"e_1_2_7_21_2","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-132-10-200005160-00005"},{"key":"e_1_2_7_22_2","first-page":"320","article-title":"Splenectomy in relapsing and plasma\u2010refractory acquired thrombotic thrombocytopenic purpura","volume":"89","author":"Kremer Hovinga JA","year":"2004","journal-title":"Haematologica"},{"key":"e_1_2_7_23_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2003.04049.x"},{"key":"e_1_2_7_24_2","doi-asserted-by":"publisher","DOI":"10.1097\/00005792-196603000-00003"},{"key":"e_1_2_7_25_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199108083250604"},{"key":"e_1_2_7_26_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199108083250605"},{"key":"e_1_2_7_27_2","first-page":"1761","article-title":"Treatment of thrombotic thrombopenic purpura. Results of a multicenter randomized clinical study","volume":"20","author":"H\u00e9non P","year":"1991","journal-title":"Presse Med"},{"key":"e_1_2_7_28_2","doi-asserted-by":"publisher","DOI":"10.1053\/j.seminhematol.2003.10.010"},{"key":"e_1_2_7_29_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1994.tb04915.x"},{"key":"e_1_2_7_30_2","doi-asserted-by":"publisher","DOI":"10.1097\/00005792-200301000-00003"},{"key":"e_1_2_7_31_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF01696619"},{"key":"e_1_2_7_32_2","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1098-1101(1998)13:3<133::AID-JCA7>3.0.CO;2-Z"},{"key":"e_1_2_7_33_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2000.02503.x"},{"key":"e_1_2_7_34_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830340303"},{"key":"e_1_2_7_35_2","doi-asserted-by":"publisher","DOI":"10.1002\/jca.2920100404"},{"key":"e_1_2_7_36_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.1996.d01-1800.x"},{"key":"e_1_2_7_37_2","doi-asserted-by":"publisher","DOI":"10.1002\/jca.1003"},{"key":"e_1_2_7_38_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2005.05418.x"},{"key":"e_1_2_7_39_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1537-2995.1998.38198141508.x"},{"key":"e_1_2_7_40_2","first-page":"838","article-title":"Therapeutic plasma exchange (TPE) for thrombotic thrombocytopenic purpura (TTP) using plasma prepared with photochemical treatment (INTERCEPT Plasma)","volume":"104","author":"Mintz PD","year":"2004","journal-title":"ASH Annual Meeting Abstracts"},{"key":"e_1_2_7_41_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.1996.d01-1836.x"},{"key":"e_1_2_7_42_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1537-2995.1999.39070748.x"},{"key":"e_1_2_7_43_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2001.02991.x"},{"key":"e_1_2_7_44_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.0042-9007.2004.00506.x"},{"key":"e_1_2_7_45_2","doi-asserted-by":"publisher","DOI":"10.1016\/S1473-0502(03)00111-3"},{"key":"e_1_2_7_46_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1537-2995.2000.40080896.x"},{"key":"e_1_2_7_47_2","doi-asserted-by":"publisher","DOI":"10.1053\/jcan.2000.18578"},{"key":"e_1_2_7_48_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1423-0410.1998.tb05479.x"},{"key":"e_1_2_7_49_2","unstructured":"The Serious Hazard of Transfusion Steering Group.Serious Hazards of Transfusion (SHOT) \u2013 Annual Report 2000\u20132001. Manchester UK SHOT 2002"},{"key":"e_1_2_7_50_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2004-07-2929"},{"key":"e_1_2_7_51_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0049-3848(02)00153-6"},{"key":"e_1_2_7_52_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.tmrv.2004.11.005"},{"key":"e_1_2_7_53_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1537-2995.2003.00571.x"},{"key":"e_1_2_7_54_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2001.02701.x"},{"key":"e_1_2_7_55_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199902113400601"},{"key":"e_1_2_7_56_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.1179"},{"key":"e_1_2_7_57_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1537-2995.2002.00260.x"},{"key":"e_1_2_7_58_2","first-page":"429","article-title":"Antiplatelet agents in thrombotic thrombocytopenic purpura (TTP). Results of a randomized multicenter trial by the Italian Cooperative Group for TTP","volume":"82","author":"Bobbio\u2010Pallavicini E","year":"1997","journal-title":"Haematologica"},{"key":"e_1_2_7_59_2","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-96-1-27"},{"key":"e_1_2_7_60_2","doi-asserted-by":"crossref","first-page":"791","DOI":"10.1001\/archinte.1992.00400160089017","article-title":"Response to plasma exchange and splenectomy in thrombotic thrombocytopenic purpura. A 10\u2010year experience at a single institution","volume":"152","author":"Onundarson PT","year":"1992","journal-title":"Arch Intern Med"},{"key":"e_1_2_7_61_2","doi-asserted-by":"crossref","first-page":"982","DOI":"10.1001\/archinte.1994.00420090056006","article-title":"Treatment outcomes in patients with adult thrombotic thrombocytopenic purpura\u2010hemolytic uremic syndrome","volume":"154","author":"Hayward CP","year":"1994","journal-title":"Arch Intern Med"},{"key":"e_1_2_7_62_2","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-122-8-199504150-00002"},{"key":"e_1_2_7_63_2","doi-asserted-by":"crossref","first-page":"302","DOI":"10.1182\/blood.V56.2.302.302","article-title":"Thrombotic thrombocytopenic purpura: a ten\u2010year experience","volume":"56","author":"Cuttner J","year":"1980","journal-title":"Blood"},{"key":"e_1_2_7_64_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2005.05681.x"},{"key":"e_1_2_7_65_2","first-page":"844","article-title":"Frequently relapsing thrombotic thrombocytopenic purpura treated with cytotoxic immunosuppressive therapy","volume":"86","author":"Allan DS","year":"2001","journal-title":"Haematologica"},{"key":"e_1_2_7_66_2","first-page":"325","article-title":"Treatment of thrombotic thrombocytopenic purpura with high\u2010dose immunoglobulins. Results in 17 patients. Italian Cooperative Group for TTP","volume":"80","author":"Centurioni R","year":"1995","journal-title":"Haematologica"},{"key":"e_1_2_7_67_2","doi-asserted-by":"publisher","DOI":"10.1007\/s002770050516"},{"key":"e_1_2_7_68_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2004.04836.x"},{"key":"e_1_2_7_69_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00277-004-0964-6"},{"key":"e_1_2_7_70_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2001.03006-3.x"},{"key":"e_1_2_7_71_2","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-128-7-199804010-00004"},{"key":"e_1_2_7_72_2","doi-asserted-by":"publisher","DOI":"10.1001\/archinte.159.21.2524"},{"key":"e_1_2_7_73_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2001.02808.x"},{"key":"e_1_2_7_74_2","doi-asserted-by":"publisher","DOI":"10.1055\/s-0037-1614408"},{"key":"e_1_2_7_75_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2002-05-1401"},{"key":"e_1_2_7_76_2","doi-asserted-by":"publisher","DOI":"10.1038\/sj.bmt.1703414"}],"container-title":["Vox Sanguinis"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fj.1423-0410.2006.00747.x","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/j.1423-0410.2006.00747.x","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,10]],"date-time":"2023-10-10T06:16:10Z","timestamp":1696918570000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/j.1423-0410.2006.00747.x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2006,2,16]]},"references-count":75,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2006,5]]}},"alternative-id":["10.1111\/j.1423-0410.2006.00747.x"],"URL":"https:\/\/doi.org\/10.1111\/j.1423-0410.2006.00747.x","archive":["Portico"],"relation":{},"ISSN":["0042-9007","1423-0410"],"issn-type":[{"value":"0042-9007","type":"print"},{"value":"1423-0410","type":"electronic"}],"subject":[],"published":{"date-parts":[[2006,2,16]]}}}